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Book ; Online: Co-curating the City

Melhuish, Clare / Benesch, Henri / Sully, Dean / Holmberg, Ingrid Martins

Universities and urban heritage past and future

2022

Keywords	City & town planning - architectural aspects ; History of architecture ; Museology & heritage studies ; Urban communities ; Higher & further education, tertiary education ; Urban & municipal planning ; urban studies ; planning ; architecture ; universities ; higher education ; heritage ; urban development ; regeneration ; built environment ; UCL ; UCLEast
Language	0\|e
Size	1 Online-Ressource
Publisher	UCL Press
Publishing place	London
Document type	Book ; Online
Note	English ; Open Access
HBZ-ID	HT021613346
ISBN	9781800081864 ; 1800081863
Database	ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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Article ; Online: Hepatoblastoma in molecularly defined, congenital diseases.

Nussbaumer, Gunther / Benesch, Martin

American journal of medical genetics. Part A

2022 Volume 188, Issue 9, Page(s) 2527–2535

Abstract: Beckwith-Wiedemann spectrum, Simpson-Golabi-Behmel syndrome, familial adenomatous polyposis and trisomy 18 are the most common congenital conditions associated with an increased incidence of hepatoblastoma (HB). In patients with these genetic disorders, ... ...

Abstract	Beckwith-Wiedemann spectrum, Simpson-Golabi-Behmel syndrome, familial adenomatous polyposis and trisomy 18 are the most common congenital conditions associated with an increased incidence of hepatoblastoma (HB). In patients with these genetic disorders, screening protocols for HB are proposed that include periodic abdominal ultrasound and measurement of alpha-fetoprotein levels. Surveillance in these children may contribute to the early detection of HB and possibly improve their chances of overall survival. Therefore, physicians must be aware of the high HB incidence in children with certain predisposing genetic diseases.
MeSH term(s)	Beckwith-Wiedemann Syndrome/diagnosis ; Beckwith-Wiedemann Syndrome/epidemiology ; Beckwith-Wiedemann Syndrome/genetics ; Child ; Genetic Diseases, X-Linked/genetics ; Gigantism/genetics ; Hepatoblastoma/diagnosis ; Hepatoblastoma/epidemiology ; Hepatoblastoma/genetics ; Humans ; Liver Neoplasms/complications ; Liver Neoplasms/diagnosis ; Liver Neoplasms/genetics
Language	English
Publishing date	2022-04-28
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2108614-X
ISSN	1552-4833 ; 0148-7299 ; 1552-4825
ISSN (online)	1552-4833
ISSN	0148-7299 ; 1552-4825
DOI	10.1002/ajmg.a.62767
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Zs.A 1376/A: Show issues

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Article ; Online: Treatment response as surrogate to predict risk for disease progression in pediatric medulloblastoma with persistent MRI lesions after first-line treatment.

Obrecht-Sturm, Denise / Schömig, Lena / Mynarek, Martin / Bison, Brigitte / Schwarz, Rudolf / Pietsch, Torsten / Pfister, Stefan M / Sill, Martin / Sturm, Dominik / Sahm, Felix / Kortmann, Rolf-Dieter / Gerber, Nicolas U / von Bueren, André O / Fleischhack, Gudrun / Schüller, Ulrich / Nussbaumer, Gunther / Benesch, Martin / Rutkowski, Stefan

Neuro-oncology

2024

Abstract: Background: This study aims at clarifying the impact of persistent residual lesions following first-line treatment for pediatric medulloblastoma.: Methods: Data on 84 pediatric patients with medulloblastoma and persistent residual lesions on ... ...

Abstract	Background: This study aims at clarifying the impact of persistent residual lesions following first-line treatment for pediatric medulloblastoma. Methods: Data on 84 pediatric patients with medulloblastoma and persistent residual lesions on centrally reviewed MRI at the end of first-line therapy were analyzed. Results: Twenty patients (23.8%) had residual lesions in the tumor bed (R+/M0), 51 (60.7%) had distant lesions (R0/M+) and 13 (15.5%) had both (R+/M+). Overall response to first-line therapy was minor or partial (≥25% reduction, MR/PR) for 64 (76.2%) and stable disease (SD) for 20 patients (23.8%). Five-year post-primary-treatment progression-free (pptPFS) and overall survival (pptOS) were superior after MR/PR (pptPFS: 62.5±7.0%[MR/PR] vs. 35.9±12.8%[SD], p=0.03; pptOS: 79.7±5.9[MR/PR] vs. 55.5±13.9[SD], p=0.04). Further, R+/M+ was associated with a higher risk for progression (5-year pptPFS: 22.9±17.9%[R+,M+] vs. 72.4±12.0%[R+,M0]; p=0.03). Watch-and-wait was pursued in 58 patients, while n=26 received additional treatments (chemotherapy only, n=19; surgery only, n=2; combined, n=3; valproic acid, n=2), and their outcomes were not superior to watch-and-wait (5-year pptPFS: 58.5±7.7% vs. 51.6±10.7% p=0.71; 5-year pptOS: 76.3±6.9% vs. 69.8±9.7%, p=0.74). For the whole cohort, five-year pptPFS by molecular subgroup (58 cases) were WNT: 100%, SHH: 50.0±35.4%, Group-4, 52.5±10.5, Group-3 54.2±13.8%; (p=0.08). Conclusion: Overall response and extent of lesions can function as surrogate parameters to predict outcomes in pediatric MB patients with persistent lesions after first-line therapy. Especially in case of solitary persistent medulloblastoma MRI lesion, additional therapy was not beneficial. Therefore, treatment response, extent/kind of residual lesions and further diagnostic information needs consideration for indication of additional treatments for persisting lesions.
Language	English
Publishing date	2024-04-05
Publishing country	England
Document type	Journal Article
ZDB-ID	2028601-6
ISSN	1523-5866 ; 1522-8517
ISSN (online)	1523-5866
ISSN	1522-8517
DOI	10.1093/neuonc/noae071
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Article ; Online: How I treat recurrent pediatric high-grade glioma (pHGG): a Europe-wide survey study.

Perwein, Thomas / Giese, Barbara / Nussbaumer, Gunther / von Bueren, André O / van Buiren, Miriam / Benesch, Martin / Kramm, Christof Maria

Journal of neuro-oncology

2023 Volume 161, Issue 3, Page(s) 525–538

Abstract: Purpose: As there is no standard of care treatment for recurrent/progressing pediatric high-grade gliomas (pHGG), we aimed to gain an overview of different treatment strategies.: Methods: In a web-based questionnaire, members of the SIOPE-BTG and the ...

Abstract	Purpose: As there is no standard of care treatment for recurrent/progressing pediatric high-grade gliomas (pHGG), we aimed to gain an overview of different treatment strategies. Methods: In a web-based questionnaire, members of the SIOPE-BTG and the GPOH were surveyed on therapeutic options in four case scenarios (children/adolescents with recurrent/progressing HGG). Results: 139 clinicians with experience in pediatric neuro-oncology from 22 European countries participated in the survey. Most respondents preferred further oncological treatment in three out of four cases and chose palliative care in one case with marked symptoms. Depending on the case, 8-92% would initiate a re-resection (preferably hemispheric pHGG), combined with molecular diagnostics. Throughout all case scenarios, 55-77% recommended (re-)irradiation, preferably local radiotherapy > 20 Gy. Most respondents would participate in clinical trials and use targeted therapy (79-99%), depending on molecular genetic findings (BRAF alterations: BRAF/MEK inhibitor, 64-88%; EGFR overexpression: anti-EGFR treatment, 46%; CDKN2A deletion: CDK inhibitor, 18%; SMARCB1 deletion: EZH2 inhibitor, 12%). 31-72% would administer chemotherapy (CCNU, 17%; PCV, 8%; temozolomide, 19%; oral etoposide/trofosfamide, 8%), and 20-69% proposed immunotherapy (checkpoint inhibitors, 30%; tumor vaccines, 16%). Depending on the individual case, respondents would also include bevacizumab (6-18%), HDAC inhibitors (4-15%), tumor-treating fields (1-26%), and intraventricular chemotherapy (4-24%). Conclusion: In each case, experts would combine conventional multimodal treatment concepts, including re-irradiation, with targeted therapy based on molecular genetic findings. International cooperative trials combining a (chemo-)therapy backbone with targeted therapy approaches for defined subgroups may help to gain valid clinical data and improve treatment in pediatric patients with recurrent/progressing HGG.
MeSH term(s)	Adolescent ; Humans ; Child ; Brain Neoplasms/therapy ; Brain Neoplasms/drug therapy ; Proto-Oncogene Proteins B-raf ; Glioma/therapy ; Glioma/drug therapy ; Temozolomide/therapeutic use ; Bevacizumab/therapeutic use ; Chronic Disease
Chemical Substances	Proto-Oncogene Proteins B-raf (EC 2.7.11.1) ; Temozolomide (YF1K15M17Y) ; Bevacizumab (2S9ZZM9Q9V)
Language	English
Publishing date	2023-02-01
Publishing country	United States
Document type	Journal Article
ZDB-ID	604875-4
ISSN	1573-7373 ; 0167-594X
ISSN (online)	1573-7373
ISSN	0167-594X
DOI	10.1007/s11060-023-04241-6
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Zs.A 1825: Show issues

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Article ; Online: Bilateral testicular juvenile granulosa cell tumor: Tumor control with conservative, antihormonal therapy.

Nussbaumer, Gunther / Benesch, Martin / Bokros, Agnes / Brecht, Ines B / Speicher, Irene / Suppan, Elisabeth / Tschauner, Sebastian / Vokuhl, Christian / Schneider, Dominik T

Pediatric blood & cancer

2022 , Page(s) e29895

Language	English
Publishing date	2022-08-04
Publishing country	United States
Document type	Letter
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.29895
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Article ; Online: Correction to: The Immune Deficiency and Dysregulation Activity (IDDA2.1 'Kaleidoscope') Score and Other Clinical Measures in Inborn Errors of Immunity.

Seidel, Markus G / Tesch, Victoria K / Yang, Linlin / Hauck, Fabian / Horn, Anna Lena / Smolle, Maria Anna / Quehenberger, Franz / Benesch, Martin

Journal of clinical immunology

2022 Volume 42, Issue 3, Page(s) 499

Language	English
Publishing date	2022-02-09
Publishing country	Netherlands
Document type	Published Erratum
ZDB-ID	779361-3
ISSN	1573-2592 ; 0271-9142
ISSN (online)	1573-2592
ISSN	0271-9142
DOI	10.1007/s10875-022-01220-w
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Zs.A 1640: Show issues

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Article ; Online: How far should we go with cost-utility analysis when treating children with acute idiopathic thrombocytopenic purpura?

Benesch, Martin

Pediatric blood & cancer

2008 Volume 50, Issue 2, Page(s) 433

MeSH term(s)	Child ; Cost-Benefit Analysis ; Decision Support Techniques ; Drug Costs ; Drug Therapy/economics ; Humans ; Immunoglobulins, Intravenous/economics ; Immunoglobulins, Intravenous/therapeutic use ; Methylprednisolone/economics ; Methylprednisolone/therapeutic use ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Purpura, Thrombocytopenic, Idiopathic/economics
Chemical Substances	Immunoglobulins, Intravenous ; Methylprednisolone (X4W7ZR7023)
Language	English
Publishing date	2008-02
Publishing country	United States
Document type	Comment ; Letter
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.21231
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Zs.A 1131: Show issues

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Article ; Online: Concurrence of a kinase-dead BRAF and an oncogenic KRAS gain-of-function mutation in juvenile xanthogranuloma.

Seidel, Markus G / Brcic, Luka / Hoefler, Gerald / Hutter, Caroline / Minkov, Milen / Steffen, Laura Sophie / Zebisch, Armin / Benesch, Martin

Pediatric blood & cancer

2022 Volume 70, Issue 4, Page(s) e30060

MeSH term(s)	Humans ; Proto-Oncogene Proteins B-raf/genetics ; Gain of Function Mutation ; Proto-Oncogene Proteins p21(ras)/genetics ; Xanthogranuloma, Juvenile/genetics ; Colorectal Neoplasms/genetics ; Mutation
Chemical Substances	Proto-Oncogene Proteins B-raf (EC 2.7.11.1) ; Proto-Oncogene Proteins p21(ras) (EC 3.6.5.2) ; KRAS protein, human ; BRAF protein, human (EC 2.7.11.1)
Language	English
Publishing date	2022-11-01
Publishing country	United States
Document type	Editorial ; Research Support, Non-U.S. Gov't
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.30060
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Article: Prognostic Impact of Pulmonary Metastasectomy in Bone Sarcoma Patients: A Retrospective, Single-Centre Study.

Smolle, Maria Anna / Kogler, Angelika / Andreou, Dimosthenis / Scheipl, Susanne / Bergovec, Marko / Castellani, Christoph / Till, Holger / Benesch, Martin / Posch, Florian / Szkandera, Joanna / Smolle-Jüttner, Freyja-Maria / Leithner, Andreas

Cancers

2023 Volume 15, Issue 6

Abstract: This retrospective study aimed at analyzing the impact of metastasectomy on post-metastasis survival (PMS) in bone sarcoma patients with lung metastases. Altogether, 47 bone sarcoma patients (24 males, median age at diagnosis of lung metastases: 21.8 ( ... ...

Abstract	This retrospective study aimed at analyzing the impact of metastasectomy on post-metastasis survival (PMS) in bone sarcoma patients with lung metastases. Altogether, 47 bone sarcoma patients (24 males, median age at diagnosis of lung metastases: 21.8 (IQR: 15.6-47.3) years) with primary (n = 8) or secondary (n = 39) lung metastases treated at a single university hospital were retrospectively included. Based on a propensity score, inverse probability of treatment weight (IPTW) was calculated to account for selection bias whether patients had undergone metastasectomy or not. The most common underlying histology was osteosarcoma (n = 37; 78.7%). Metastasectomy was performed in 39 patients (83.0%). Younger patients (
Language	English
Publishing date	2023-03-13
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers15061733
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Article: Rapidly involuting congenital hemangioma of the liver in a newborn with incomplete Pentalogy of Cantrell: description of a new association.

Gasparella, Paolo / Singer, Georg / Arneitz, Christoph / Benesch, Martin / Sorantin, Erich / Kampelmühler, Eva / Schemmer, Peter / Till, Holger

Journal of surgical case reports

2021 Volume 2021, Issue 3, Page(s) rjab047

Abstract: The perinatal management of newborns with giant omphaloceles requires careful suspicion concerning 'unexpected' associated malformations. We describe a newborn with a combination of incomplete Pentalogy of Cantrell consisting of giant omphalocele, ... ...

Abstract	The perinatal management of newborns with giant omphaloceles requires careful suspicion concerning 'unexpected' associated malformations. We describe a newborn with a combination of incomplete Pentalogy of Cantrell consisting of giant omphalocele, anterior congenital diaphragmatic hernia (CDH) and pericardial absence complicated by a hepatic rapidly involuting congenital hemangioma (RICH). A giant omphalocele was detected prenatally. Postnatally, staged closure of the omphalocele was planned. A mass of the liver was noted and (mis-)taken for a hematoma. In the further course, the baby developed cardiorespiratory insufficiency due to a central CDH which was excised. Subsequently, staged closure of the omphalocele became impossible. Specific workup revealed a mass in the left liver lobe. The mass was resected and the abdominal wall defect repaired. Histology confirmed the diagnosis of an RICH. The surgical treatment of newborns with giant omphaloceles requires a multidisciplinary neonatal support and an elaborate pediatric surgical armamentarium to cope with additional malformations.
Language	English
Publishing date	2021-03-22
Publishing country	England
Document type	Case Reports
ISSN	2042-8812
ISSN	2042-8812
DOI	10.1093/jscr/rjab047
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